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Spinal Cord Compression by Tophi in a Patient with Chronic PolyarthritisCase Report and Literature Review.

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1431
BRIEF REPORT
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... _.
SPINAL CORD COMPRESSION BY TOPHI IN A PATIENT WITH
CHRONIC POLYARTHRITIS:
CASE REPORT AND LITERATURE REVIEW
S T E V E N K. MAGID, G E O R G E E. GRAY. and AZAD A N A N D
Nervous system involvement in tophaceous
gout is rare. Case reports of median nerve compression by tophi have been published (1). To date, compression of the spinal cord by tophi has been described
only once before (2). We report a patient with spinal
cord compression by tophaceous masses and evidence
of median nerve entrapment and cervical radiculopathy on a similar basis.
Case Report. A 50-year-old white postmenopausal woman was admitted for evaluation of back
pain and lower extremity numbness and weakness of 3
weeks’ duration. At age 1 1, she had a 3-week episode
of left elbow arthritis. She was asymptomatic until
approximately age 21, when she had the onset of
severe intermittent bilateral ankle pain. By age 26, the
wrists, knees, temperomandibular joints, and toes
were involved. Rheumatoid arthritis (RA) was diagnosed, and the patient was treated with antimalarials,
injections of gold salts, aspirin, and several nonsteroidal antiinflammatory drugs. Steroids were used intermittently for 25 years and continuously for 8 years
before admission. She is currently taking prednisone
15-20 mg orally per day.
In the year before her admission, flexion contractures of multiple digits developed, and punctate
white lesions appeared on her elbows, forearms, buttocks, and chest. Two months before admission, she
__
From the Division of Rheumatic Diseases, Department of
Medicine, The Hospital for Special Surgery, The New York Hospital-Come11 University Medical Center, and the Departments of
Pathology and Radiology, The New York Hospital-Come11 University Medical Center, New York, NY 10021.
Steven K. Magid. MD; George E. Gray, MD, Azad Anand,
MD .
Address reprint requests to I h . Steven K . Magid. The
IIospital for Special Surgery, 535 East 70th Street. New York, NY
10021.
Submitted for publication February 18. 1981; accepted in
revised form July 8, 1981.
Arthritis and Rheumatism, Vol. 24, No. 11 (November 1981)
noted lower extremity weakness, followed by an increase in chronic midthoracic back pain. Two days
before admission, the patient noted numbness below
the level of the breast and became totally unable to
ambulate. Bowel and bladder dysfunction occurred.
Additional medications at the time of admission
were h ydrochlorothiazide and propranolol for longstanding hypertension. There was no family history of
gout, kidney stones, inflammatory arthritis, or mental
deficiency. The patient’s father had renal insufficiency
of unknown etiology. There was no unusual dietary
history, no known lead or toxin exposure, and only
moderate alcohol consumption.
Physical examination on admission revealed a
woman with cushingoid facies. The blood pressure
was 170/100, and pulse was 100. Examination showed
that her lungs, heart, and abdomen were normal.
Crops of pustule-like skin lesions were present over
the arms, chest, and legs (Figure 1). These lesions
contained chalky-white liquid that, when examined
with polarized light, was packed with negatively birefringent, needle-shaped crystals. Small tophi were
present in the ears. Sensation was decreased below
T6-8. There was marked weakness of the lower extremities, accompanied by absent patellar and achilles
deep-tendon reflexes and bilateral Babinski signs.
Loss of sensation occurred in both median and ulnar
distribution distal to the right wrist. A 1.5 x 2 cm mass
was noted on the volar surface of the right wrist.
Tinel’s sign was present, and a right-sided C6-7 sensory defect was noted. There was decreased range of
motion of all proximal interphalangeal, distal interphalangeal, and metacarpophalangeal joints, as well as
both wrists, but elbows and shoulders were normal.
Bilateral flexion contractures of the knee were present, and ankle motion was severely limited and
painful.
1432
BRIEF REPORTS
Blood uric acid by autoanalyzer was 11.2 mgldl,
and creatinine clearance was 37.5 muminute with a 24hour urinary creatinine of 879 mg. Urinary “total uric
acid” (uric acid plus oxypurine) was 544 mglday (while
on 300 mg of allopurinol for I day). The following red
blood cell enzyme determinations, kindly performed
by Dr. E. W. Holmes, were normal: hypoxanthine
phosphoribosyltransferase (PRT), guanine PRT. adenine PRT, and PRPP syntheses.
Other laboratory values included: an initial leukocyte count of 18,000 mm3, hemoglobin of 14.9 gm,
hematocrit of 41%, and erythrocyte Sedimentation rate
63 mm (Westergren). The cerebrospinal fluid (CSF)
was clear and colorless, with 1 red blood cell/mm3 and
I white blood cell/mm3 (100% lymphocytes and mononuclear cells). The CSF protein was 659 mg%, and the
CSF glucose was 89 mg%. Results of all CSF cultures
were negative. The antinuclear antibody was 2-3+
speckled, and DNA binding (Farr assay) was 3%
(<20%). The CH50 was 240 (normal 150-200). and the
results of latex slide agglutination test were negative.
Figure 1. Extensor surface of elbow. Note the multiple small tophi,
some of which have a surrounding area of erythema.
When she was admitted, a Pantopaque myelogram was performed via Cl-2 and L4-5 punctures. A
total block extended from T2 to T8-9 (Figure 2). The
subarachnoid space in the lowcr thoracic and lower
cervical region was irregular, and there was a localized
epidural defect at C7.
A decompression laminectomy was performed
from TI-9 on the day of admission. Thick “caseous”
material was obtained from what appeared to be an
epidural abscess, but Gram stain and Ziehl Neelsen of
this material did not reveal bacteria. Operative specimens were fixed in formalin and routinely processed.
Examination of histologic sections revealed a chronic
inflammatory cellular infiltrate with multinucleated
giant cells surrounding eosinophilic, amorphous material consistent with a tophus (Figure 3). Crystals
typical Of uric acid were apparent On unfixed tissue
pressed Onto a glass slide and examined with POlariZing lenses.
Figure 2. Pantopaque myelogram. Note the total obstruction to
flow at 1-2 and 1-8-9 and irregular filling defects on fight bide of
lowcr cervical region.
BRIEF REPORTS
Figure 3. Gouty tophus from extrddural soft tissue. Mononuclear
cells and multinucleated foreign body-type giant cells surround
proteinaceous ground substance from which uric acid crystals have
been dissolved by tissue-processing solutions. This is the typical
histopathologic appearance of a tophus. (Hemotoxylin and eosin:
magnification ~ 3 2 . )
The HLA type was A l , B8, -, DRw6/A28, B51,-, DRl
(kindly performed by Drs. Gibofsky and Fotino).
Radiographs of the hands showed bilateral destruction of the carpal bones, generalized demineralization, an area of pronounced demineralization of the
distal right radius, and an erosion of the right trapezium; feet roentgenograms showed narrowing of the
tarsal joints and bony fusions, but metatarsophalangeal joints were not involved. Severe medial and
lateral compartment narrowing accompanied by marginal erosion was apparent in knee radiographs, and
cervical spine films revealed anterior subluxation of
CI and C2. The odontoid was not well visualized.
After surgery, there was gradual improvement
in lower extremity strength and a marked decrease in
back pain. Allopurinol was prescribed, and, at the time
of her discharge from the hospital, her uric acid had
decreased to 7.3 mg/dl. Colchicine was started because of recurrent joint pain and arachnoiditis. Four
months after surgery, the patient is able to ambulate
with assistance. She has subsequently undergone both
a right wrist tophectomy and a carpal tunnel release.
Discussion. Spinal cord compression by tophaceous deposits has been reported only once in the past
(2): a 44-year-old man who had a 5-month history of
midthoracic back pain, lower extremity paralysis, sensory loss, and bladder and bowel dysfunction. A
laminectomy was performed, and a friable chalkywhite mass (histologically and chemically identified as
uric acid) extended dorsolaterally from T9 to TI1 in
1433
the spinal canal. Two other cases of back and lower
extremity pain and paresis and sensory abnormalities
have been reported secondary to tophaceous masses
impinging on the cauda equina (3,4).
In addition, asymptomatic spinal involvement
was noted at autopsy in 2 other subjects, 1 in whom
deposits of urate in the intervertebral discs and adjacent vertebral bodies were found (5). In the other,
facet joints and adjacent ligaments of L4-5 and LS-Sl
were extensively involved by tophi (6). Erosion and
subluxation in the cervical spine, a well known complication of RA, may also occur in gout. Two such
cases have been reported in patients with long-standing tophaceous gout who presented with acute neck
pain (73).
The present case is interesting for a number of
reasons. In addition to spinal cord compression by
tophi, a rare complication of gout, the patient had two
other neyrologic defects caused by tophi-a C6 radiculopathy and carpal tunnel syndrome. The early age of
onset of gouty arthritis in this patient is very unusual.
Although no enzyme defect, genetic predisposition, or
hematologic process could be identified in this patient,
obesity, hypertension, and diuretic use may have
contributed to the clinical presentation.
This case also demonstrates dramatically the
difficulty that may occur in differentiating tophaceous
gout from RA. Many features of gout may mimic RA
(9), including symmetric polyarthritis, fusiform swelling, and subcutaneous nodules. Spinal involvement
has also been described in both diseases. RA causes
erosive disease of the cervical spine, and rheumatoid
nodules may affect vital neural structures at other
levels of the spinal axis (10). Analogous processes
occur in gout (see above). While many features of this
patient’s illness indicate the coexistent presence of RA
(the onset of disease in a young woman, Cl-C2
subluxation, symmetric involvement of joints, fusiform joint swelling, and the severe destructive
changes in the carpal bones that radiographically were
most suggestive of RA), a definite diagnosis of RA
cannot be made. The case, however, serves to emphasize the need for rigorous examination of synovial fluid
for uric acid crystals in those patients who may have
RA. Compression of neural structures by tophi is most
often identified in patients with long-standing, polyarticular, tophaceous gout, and, when neurologic symptoms occur in this patient population, this diagnosis
must be considered. Surgical decompression with subsequent pharmacologic attempts to mobilize tophi may
result in normal or improved function.
BRIEF REPORTS
1434
ACKNOWLEDGMENTS
We wish to thank Drs. M. Lockshin, B. Sigsbee, R.
A. R. Fraser, and L. R. Straub for allowing us to study their
patient, and Mrs. Hjordis Kerezman for her help in preparing this manuscript.
5.
6.
7.
REFERENCES
Ward LE, Bickel WH, Corbin KB: Median neuritis
(carpal tunnel syndrome) caused by gouty tophi. JAMA
167~844-846, 1958
Koskoff YD, Morris LE, Lubic LG: Paraplegia as a
complication of gout. JAMA 152:37-38, 1953
Litvak J, Briney W: Extradural spinal depositions of
urates producing paraplegia. J Neurosurg 39:656-658,
1973
Reynolds AF, Wyler AR, Nonis HT: Paraparesis sec-
8.
9.
10.
ondary to sodium urate deposits in the ligamentum
flavum. Arch Neurol 33:795, 1976
Lichtenstein L, Scott HW, Levin MH: Pathologic
changes in gout. Am J Pathol 32:871-895, 1956
Hall MC, Selin G: Spinal involvement in gout. J Bone
Joint Surg 42-A:341-343, 1960
Kersley GD, Mandel L, Jeffrey MR: Gout: an unusual
case with softening and subluxation of the first cervical
vertebra and splenomegaly. Ann Rheum Dis 9:282-303,
1950
Vinstein AL, Cockerill EM: Involvement of the spine in
gout. Radiology 103:311-312, 1972
Talbott JH, Altman RD, Yu TF: Gouty arthritis masquerading as rheumatoid arthritis or vice versa. Semin
Arthritis Rheum 8:77-114, 1978
Markenson JA, McDougal JS, Tsairis P, Lockshin MD,
Christian CL: Rheumatoid meningitis: a localized immune process. Ann Intern Med 90:78&789, 1979
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spina, report, patients, polyarthritiscase, literatury, cord, compression, review, chronic, tophi
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